Lymphoma

Question 1

Define Lymphoma (3)

Answer 1

Solid cohesive neoplasms of LYMPHOID TISSUE: often LNs, sometimes other LNs - spleen/MALT, rarely anywhere e.g. skin, CNS, breast

Question 2

Hodgkin’s lymphoma: epidemiology (2), virus association (1), LN spread (1)

Answer 2

M>F & bi-modal age incidence - 20-29 & >60 EBV associated Contiguous spread to LNs (follows efferent route)

Question 3

Hodgkin’s lymphoma: Clinical presentation (4)

Answer 3

Painless asymmetrical lymphadenopathy +/- obstructive/mass effect symptoms Pain in nodes after alcohol Pel-Ebstein fever - cyclical 1-2 week (minority) B symptoms may be present (due to effect of metabolic load of tumour producing cytokines effecting hypothalamus) - wt loss, night sweats, low fever etc.

Question 4

Hodgkin’s lymphoma: Ix - imaging (2), tissue diagnosis (biopsy shows 2 markers), Buzz word cell, subtypes (5)

Answer 4

CT/PET Biopsy shows CD 15 or 30 Reed-sternberg cell: bi-nucleate (owl-eyed) cell on background of lymphocytes and reactive cells Subtypes: nodular sclerosing, mixed cellularity, lymphocyte rich, lymphocyte depleted, nodular lymphocyte predominant

Question 5

Staging of Hodgkin’s lymphoma (4)

Answer 5

Stage 1: 1 LN region Stage 2: 2 or more LN regions on same side of diaphragm stage 3: 2 or more LNs on opposite sides of diaphragm Stage 4: extranodal sites e.g. liver/ BM A = no constitutional sym B= constitutional sym

Question 6

Management of Hodgkin’s lymphoma: 3 categories

Answer 6

Combination chemo: > most cases, ABVD: adriamycin, Bleomycin, vinblastine, dacarbazine > 2-4 cycles in stage 1/2, 6-8 if > stage 2 Radiotherapy Intensive chemo + autologous SCT - used in pts with relapse

Question 7

Stem cells can be harvested from 3 sources - what are they?

Answer 7

Peripheral blood following stimulation by G-CSF BM Umbilical cord blood

Question 8

Briefly describe the process of autologous SCT (4) and its benefits (2)

Answer 8

Pts own SCs harvested High dose chemo to eradicate malignant cells +/- BM Frozen SCs reintroduced in pt used more in multiple myeloma & lymphoma than in leukaemia Benefits - no GvHD, & lower risk of infection

Question 9

Briefly describe the process of allogeneic SCT (4) and its risks (4)

Answer 9

HLA-matched donor SCs are harvested Pts BM eradicated by chemo Donor SCs introduced and colonise ‘empty’ BM Used more in leukaemia Risks: GvHD, opportunistic infections, infertility, secondary malignancies

Question 10

Non-hodgkin’s lymphoma (80%), 40 diff subtypes, can be classfied based on maturity(2), histology (2), and lineage (2)

Answer 10

Mature or immature High grade - v. aggressive e.g. burkitts low grade - indolent e.g. follicular, marginal zone, Lineage - B or T cell

Question 11

Follicular lymphomas: Grade, response to treatment, translocation,

Answer 11

Indolent But poor response to treatment (this is because of the anti-apoptotic property of BCL-2 - from translocation) T( 14:18) - BCL-2 translocates next to Ig gene (hotspot) > over expression of BCL-2 proteins .

Question 12

Follicular lymphomas: histology (2), treatment (2)

Answer 12

Follicular pattern Nodular appearance treatment - watch and wait, rituximab (these tumours would probably have undergone hypermutation)

Question 13

Mantle cell lymphoma: epidemiology (2), Grade, translocation & involved gene

Answer 13

Middle aged, M> F Aggressive T (11;14) - overexpression of Cyclin D1

Question 14

Mantle cell lymphoma: histology (1), treatment (2)

Answer 14

Angular nuclei treatment - rituximab-CHOP, auto-SCT for relapse

Question 15

Diffuse large B cell lymphoma (DLBL): epidemiology, grade,

Answer 15

Middle aged and elderly (most common NH lymphoma) Aggresive other lymphomas occur secondary to DLBL a large percentage show 18:14 translocation (prev follicular) EBV association e.g. in HIV or post transplant

Question 16

DLBL: histology (1), treatment (2)

Answer 16

“Sheets of large lymphoid cells” Rituximab-CHOP Auto-SCT for relapse

Question 17

Burkitt’s lymphoma: general description- grade (1), translocation + involved gene, response to treatment (1), histology (1), treatment (2)

Answer 17

VERY aggressive Due to nature of translocation (8;14) - myc gene translocates to Ig hotspot > overexpression of myc > uncontrolled rapid proliferation of cell rapidly responsive to treatment

Histology: “starry sky” appearance ( this is because rapid cell proliferation overloaded with chromatin - super dark. High rate of mitoses & apoptosis - infiltration of macrophages - star shaped, small nuclei with pale cytoplasm so prominent contrast with dark neoplastic cells)

Treatment: rituximab (anti-CD20), SCT

Question 18

Burkitt’s lymphoma: endemic subtype characteristics: epidemiology (1), viral association (1), anatomical involvement (1)

Answer 18

Most common malignancy in equatorial AFRICA 100% EBV association JAW involvement

Question 19

Burkitt’s lymphoma: sporadic subtype characteristics

Answer 19

Outside Africa (america) 20% associated with EBV Abdo cavity more involved

Question 20

Burkitt’s lymphoma: immuno-def pts, 2 characteritics

Answer 20

Non-EBV associated HIV/ post-transplant pts

Question 21

MALTomas/ marginal zone lymphomas: why do these occur? (1) 2 examples, treatment principle(1)

Answer 21

Due to CHRONIC ANTIGEN STIMULATION:

SJOGREN’S - parotid lymphoma

H. PYLORI - Gastric MALT lymphoma

treat by removing ag stimulus (some regress just by that; because the lack of ag stim to H. pylori specific T cells means it stops producing cytoines which support the lymphoma)

Question 22

T- cell lymphomas name 5 types and what agent is used in treatment for all

Answer 22

anaplastic large cell lymphoma

peripheral T-cell lymphoma

adult T cell leukaemia/lymhoma

Enteropathy-associated T cell lymhoma (EATL)

Cutaneous T cell lymphoma

Treat - Alemtuxumab (anti-CD52)

Question 23

Anaplastic large cell lymphoma: epidemiology (1),grade, histology (1), translocation and involved protein

Answer 23

Children and young adults

aggressive large ‘epitheliod’ lymphocytes

t(2;5) - Alk-1 protein expression

Question 24

Peripheral T cell lymphoma: epidemiology, grade, histology

Answer 24

Middle-aged & elderly aggressive large T-cells

Question 25

Adult T cell leukaemia/lymphoma: epidemiology (countries), viral association, grade

Answer 25

Carribean & Japanese HTLV-1 Aggressive

Question 26

Enteropathy associated T cell lymphoma (EATL) - main association (1)

Answer 26

Coeliac disease

Question 27

Cutaenous T cell lymphoma - main association (1)

Answer 27

Mycosis fungoides